Details

Autor(en) / Beteiligte

• Emmungil, H.
• Erden, A.
• Küçükşahin, O.
• Zengin, O.
• Ermurat, S.
• Gök, K.
• Doğru, A.
• Kalfa, M.
• Akyol, L.
• Erbasan, F.
• Çınar, M.
• Balcı, M.A.
• Yazıcı, A.
• Yavuz, S.
• Göker, B.
• Sarı, A.
• Turgay, M.
• Onat, A.M.
• Kısacık, B.
• Pehlivan, Y.
• Şenel, S.
• Şahin, M.
• Sayarlıoğlu, M.
• Pamuk, Ö.N.
• Çefle, A.
• Aydın, S.Z.
• Kalyoncu, U.

Titel

FRI0373 The Clinical and Demographic Features of Relapsing Polychondritis: A Nationwide Study

Ist Teil von

• Annals of the rheumatic diseases, 2016-06, Vol.75 (Suppl 2), p.569

Ort / Verlag

London: BMJ Publishing Group LTD

Erscheinungsjahr

2016

Quelle

BMJ Journals Archiv - DFG Nationallizenzen

Beschreibungen/Notizen

• BackgroundRelapsing polychondritis (RP) is a rare systemic and inflammatory disease.Objectivesto assess clinical features, management and prognosis in RP.MethodsRetrospective medical records were collected from 16 centers for 37 patients with RP diagnosed by Michet criteria. Data included patients' demographics, clinical features, laboratory findings and outcome.ResultsA total of 37 patients, (Male/female=19/18) with a mean disease onset of 45.7±11.8 years and median follow up period is 20 (1–360) months were enrolled. The most common initial complaints and organs involved were showed in table 1. Mean lead time to diagnose was 28.6±54.3 (0–228) months. Four patients had a known history of cartilage injury before the RP diagnosis (Three goiter operation and 1 resuscitation due to a drowning). Twelve of the patients (32.4%) had also various autoimmune or hematologic diseases. After the diagnosis, moderate or high dose prednisone (n=37, 100%), plus methotrexate (n=15, 40.5%) or azathioprine (AZA) (n=11, 29.7%) or hydroxychloroquine (HQ) (n=3, 8.1%) or cyclophosphamide (CYP) (n=2, 5.4%), or (AZA+ HQ n=3, 8.1%) treatments were initiated. In the case of resistant disease, CYP (n=6, %16.2), tocilizumab (n=2, 5.4%), etanercept (n=1, 2.7%), infliximab (n=1, 2.7%) and bevacizumab (n=1, 2.7%) treatments were given. Due to tracheal stenosis surgery applied to 2 patients, one patient died due to respiratory collapse, 21 patients on remission without any complications, 13 (35.1%) patients had developed tracheal stenosis (n=5, 13.5%), major airway cartilage loss (n=5, 13.5%), auricular collapse (n=2, 5.4%), nasal collapse (n=3, 8.1%), subglottic stenosis (n=2, 5.4%), hearing loss (n=5, 13.5%), vision loss (n=1, 2.7%) and deforming arthritis (n=1, 2.7%). In the univariate analysis, only major airway involvement had a relationship with complication development. (OR 12, 95% CI 1.97–72.8, p=0.007)Table 1.Clinical features at diagnosisComplaintsn (%)Organ involvementn (%)Pain and redness in the auricular area32 (86.4)Auricular chondritis32 (86.4)Constitutional symptoms30 (81.8)Arthritis22 (59.4)Arthralgia28 (75.7)Eye19 (51.3)Swollen joints22 (59.4)Skin lesions14 (37.8)Myalgia21 (56.8)Nasal chondritis11 (29.7)Red eye and pain20 (54.1)Major airway10 (27)Erythema and pain in the nose16 (43.2)Costochondritis9 (24.3)Dyspnea13 (35.1)Hearing loss7 (18.9)ConclusionsRP is a rare disease which diagnosis is commonly delayed and lead to cartilage loss. RP developed after trauma in 4 patients, possibly trauma can be blamed as an etiologic factor. Awareness and early diagnosis results with better prognosisDisclosure of InterestNone declared